{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,31]],"date-time":"2026-07-31T18:10:23Z","timestamp":1785521423726,"version":"3.56.0"},"reference-count":42,"publisher":"Oxford University Press (OUP)","issue":"7","license":[{"start":{"date-parts":[[2026,7,31]],"date-time":"2026-07-31T00:00:00Z","timestamp":1785456000000},"content-version":"vor","delay-in-days":30,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Vaincre la Mucoviscidose","award":["RC20180502225"],"award-info":[{"award-number":["RC20180502225"]}]},{"name":"Vaincre la Mucoviscidose","award":["RC20210502815"],"award-info":[{"award-number":["RC20210502815"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,7,10]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>A key aspect of cystic fibrosis pathophysiology is the significant role played by neutrophils, central to the inflammatory response in cystic fibrosis airways. Neutrophil-dominated inflammation greatly influences clinical outcomes, including airway damage and lung function decline. People with cystic fibrosis have an increase in circulating neutrophils that exhibit numerous functional and phenotypic abnormalities.<\/jats:p>\n                  <jats:p>We used spectral flow cytometry to delineate the expression of 24 cell surface markers on neutrophils from 24 healthy donors and 45 adults with cystic fibrosis, before and after elexacaftor-tezacaftor-ivacaftor treatment. This comprehensive analysis examined phenotypic markers associated with fundamental neutrophil functions, including differentiation, maturity, activation, antimicrobial activities (degranulation, pathogen sensing and chemotaxis), metabolism, and immunomodulation.<\/jats:p>\n                  <jats:p>Prior to treatment, neutrophils from adults with cystic fibrosis displayed a phenotype suggestive of immune activation and metabolic aberration. Treatment with elexacaftor-tezacaftor-ivacaftor normalized a FPR1high\/CXCR2high subset, an observation consistent with enhanced pathogen-sensing capacity. The treatment, however, failed to restore fully normal phenotypes in cystic fibrosis neutrophils but rather induced the modulation of a PD-L1high\/CD114high\/GLUT-1high subset of mature neutrophils with an increased expression of PD-L1 and adhesion molecules such as CD11c and CD11b.<\/jats:p>\n                  <jats:p>Taken together, our data reveal a specific immunophenotypic signature of neutrophils in cystic fibrosis that is further modified by treatment with elexacaftor-tezacaftor-ivacaftor, suggesting the opportunity for developing adjunctive therapies to enhance beneficial antimicrobial subsets while simultaneously mitigating immunosuppressive phenotypes, thereby opening new perspectives for immune modulation in cystic fibrosis treatment.<\/jats:p>","DOI":"10.1093\/immhor\/vlag030","type":"journal-article","created":{"date-parts":[[2026,7,31]],"date-time":"2026-07-31T17:01:56Z","timestamp":1785517316000},"source":"Crossref","is-referenced-by-count":0,"title":["CFTR modulators exert subset-specific phenotype remodeling on circulating neutrophils in cystic fibrosis"],"prefix":"10.1093","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0009-0008-2502-526X","authenticated-orcid":false,"given":"Fran\u00e7ois","family":"Chable de la H\u00e9ronni\u00e8re","sequence":"first","affiliation":[{"name":"Inserm U1016, Institut Cochin , Paris,","place":["France"]},{"name":"Universit\u00e9 Paris-Cit\u00e9 , Paris,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Th\u00e9o","family":"Dh\u00f4te","sequence":"additional","affiliation":[{"name":"Inserm U1016, Institut Cochin , Paris,","place":["France"]},{"name":"Universit\u00e9 Paris-Cit\u00e9 , Paris,","place":["France"]},{"name":"Service de Pneumologie & Centre de R\u00e9f\u00e9rence Maladies Rares Mucoviscidose, site coordonnateur, H\u00f4pital Cochin, Assistance Publique-H\u00f4pitaux de Paris-Centre & Universit\u00e9 Paris-Cit\u00e9 , Paris,","place":["France"]},{"name":"ERN-Lung, 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